AB0650 Lung Manifestations of Diffuse Cutaneous Systemic Sclerosis Patients from India
Authors: A. Samitinjay, S. N. Vithala
Journal: Annals of the Rheumatic Diseases (2022)

Background Lung manifestations in diffuse cutaneous systemic sclerosis (dCSS) are well known. Data on these manifestations in Indian patients are scant or unknown. We aim to present the various lung manifestations in patients with dCSS. Objectives To study the clinical and radiological characteristics of pulmonary symptoms such as cough, dyspnea, or hemoptysis, in patients, previously diagnosed with dCSS, in accordance with ACR 2013 classification criteria. Methods This was a cross-sectional, point of care study, done at the Government General and Chest Hospital, Hyderabad, Telangana, India. The study was conducted from April 2019 to March 2020 and patients with a confirmed diagnosis of dCSS were included. Patients with known heart disease, diabetes mellitus, cancer, obesity, acute respiratory infections, and HIV infection were excluded. 7 patients were eligible for inclusion. All patients gave their consent to participate in this study. The study was also approved by the institutional ethics committee. All imaging studies were read and confirmed by consultant radiologists at our hospital. Results All patients received a confirmed diagnosis of dCSS less than 2 years from the time of presentation to our hospital. The shortest duration from diagnosis of dCSS to first manifestation of pulmonary symptoms was 6 months. All patients were females. The mean age was 52 ± 14.6 years. All patients had chronic cough, lasting ≥ 1 month. 6 out of 7 patients had exertional dyspnea lasting for ≥ 1 month. None of the 7 patients reported hemoptysis, wheezing, or having a fever. Only 1 patient had anemia, and none had leukocytosis. All patients had their chest x-rays reported as normal. 6/7 (85.7%) patients had subpleural reticular shadowing limited to the lower lobes on HRCT chest, consistent with usual interstitial pneumonia (UIP) pattern. 3/6 (50%) patients with reticular shadowing also had honeycombing suggesting advanced/severe UIP. None of the patients had traction bronchiectasis. 2/6 patients (33.3%) with UIP, also had non subpleural ground glass opacities (GGOs) in the middle and lower zones, suggesting an overlap between UIP and NSIP (non-specific interstitial pneumonia). Findings on ECG, 2D-Echo, spirometry and bronchoalveolar lavage (BAL) are summarized in Table 1. Table 1. S.No. Age (years) Sex Duration from Diagnosis to first pulmonary manifestation Pulmonary symptoms CBC Chest X-ray HRCT Chest ECG 2D-Echo Spirometry BAL Patient 1 44 F 16 months Cough + Dyspnea – 2 months Normal Normal Advanced UIP Normal Normal Restrictive Normal Patient 2 56 F 18 months Cough + Dyspnea – 3 months Normal Normal UIP Normal Normal Normal Neutrophilic Patient 3 61 F 12 months Cough + Dyspnea – 1 month Normal Normal Advanced UIP Normal RVSP = 48 mm Hg Restrictive Lymphocytic Patient 4 70 F 21 months Cough + Dyspnea – 2 months Normal Normal Normal Normal Normal Normal Lymphocytic Patient 5 24 F 18 months Cough + Dyspnea – 2 months Normal Normal Advanced UIP + NSIP (overlap) Normal RVSP = 51 mm Hg Restrictive Neutrophilic Patient 6 55 F 12 months Cough + Dyspnea – 1 month Normal Normal UIP Normal Normal Normal Not Performed Patient 7 54 F 6 months Dyspnea – 1 month Anemia + Normal UIP + NSIP (overlap) Normal Normal Restrictive Neutrophilic Notes in the column ‘BAL’ indicate the predominant cell type noted on cytology. RVSP = Right Ventricular Systolic Pressure Conclusion Although limited by a small sample size, UIP appeared to be the most common lung pathology in patients with dCSS. The duration from diagnosis of dCSS to first manifestation of pulmonary symptoms is much shorter in comparison to limited cutaneous systemic sclerosis [1] PAH is commoner with limited cutaneous systemic sclerosis than with dCSS. [2] BAL was neither useful for diagnosis nor required in preparing a therapeutic plan for these patients. References [1]Steen VD. Clinical manifestations of systemic sclerosis. InSeminars in cutaneous medicine and surgery 1998 Mar 1 (Vol. 17, No. 1, pp. 48-54). [2]Mathai S, Kroening K, Hummers L, Zaiman A, Girgis R, Hassoun P. Differences between limited and diffuse systemic sclerosis-related pulmonary arterial hypertension. Disclosure of Interests None declared